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At least 613 records · Page 34Linked to original sources
PubMed1988

[Monostotic fibrous dysplasia of the sphenoidal bone presenting with visual disturbance during pregnancy].

A case of monostotic fibrous dysplasia in the anterior skull base, which presented with visual disturbance during pregnancy, is reported. A 32-year-old female was referred to our department for examination of the progressing right visual disturbance in the third trimester of her second pregnancy. She had experienced the same episode in her first pregnancy, recovering from it after delivery. This time, however, the visual acuity did not change after delivery. Plain craniogram showed sclerotic changes in the right sphenoidal ridge and the right frontal skull base. CT scan showed an isodense mass which was enhanced by contrast medium in the right sphenoidal sinus. Angiography demonstrated no positive findings. RI bone scintigram using 99 m Tc-MDP revealed an abnormal uptake in this region. The patient was operated on in two stages. The first operation was transsphenoidal removal of the tumor in the sphenoidal sinus. The pathological diagnosis was fibrous dysplasia. Transfrontal decompression of the right optic canal and ophthalmic artery was performed at the second operation. The tumor was totally removed and the decompressed orbital roof was reconstructed using an alumina ceramic plate. Visual acuity gradually improved in the follow-up study. To our best knowledge, only one case of fibrous dysplasia with growth during pregnancy have been reported; it was monostotic fibrous dysplasia of the maxilla. From the clinical course of our case, it is suggested that the pregnancy influenced growth of the monostotic fibrous dysplasia; possibly by way of hypothalamic hormonal factor.

Female↗
PubMed1987

Brushite in fibrous dysplasia of the jaw bone.

Crystals associated with the maxillary bone in a case of fibrous dysplasia were investigated histopathologically, crystallo-graphically and by infrared absorption spectroscopy. Light microscopically, the crystals showed a transparent and plate-like appearance, and were revealed to be piled, incomplete monoclinic structures up to 250 micron in size by scanning electron microscopy. These crystals were identified as brushite, dicalcium phosphate dihydrate CaHPO4.2H2O, by X-ray microdiffraction and infrared absorption spectroscopic analysis. From consideration of the equilibrium of brushite, it was assumed in this case that inflammation had occurred initially and that the tissue fluid conditions at the site of the lesion had deviated from normal, eventually resulting in the deposition of brushite crystals on the bone apatite. From a crystallographic view point, the mode of occurrence of brushite suggested that the bone at the lesion site would not recover through normal healing.

Adolescent↗
PubMed2002

Bone mineralization in polyostotic fibrous dysplasia: histomorphometric analysis.

Fibrous dysplasia (FD) of bone can be complicated by renal phosphate wasting. The effect of hypophosphatemia on normal and dysplastic bone of FD patients has not been well characterized. In this study, we compared serum phosphorus (sPi) levels to histomorphometric findings in 27 iliac bone samples from 23 children and adolescents (aged 4.2-16.4 years) with polyostotic FD. The samples were separated into two groups, based on the presence (n = 10) or absence (n = 17) of a dysplastic lesion within the specimen. Histomorphometric results were compared with those from 18 age-matched control subjects without metabolic bone disease. In dysplastic lesions, trabeculae were clearly thinner and increased in number. Osteoid indices, osteoblast surface per bone surface, and mineralization lag time were elevated in dysplastic areas, but there was no detectable effect of sPi concentrations on these indices. In nondysplastic bone tissue, low sPi levels were associated with mildly increased osteoid thickness and prolonged mineralization lag time. None of the 13 patients in whom hand X-rays were available at the time of biopsy had radiological signs of rickets. In conclusion, low sPi can cause a mild systemic mineralization defect in FD, but the more severe mineralization defect seen in dysplastic lesions is independent of sPi levels. It is debatable whether the mild systemic mineralization defect warrants treatment with oral phosphorus supplementation if signs of rickets are absent.

Adolescent↗
PubMed2004

Surgery improves vision and cosmetic appearance of an adult patient with fibrous dysplasia of the frontal bone.

A 31-year-old woman with fibrous dysplasia (FD) of the left forehead was reported. Visual acuity impairment and diplopia were slowly progressive for 6 months associated with marked protrusion of her left forehead. Removal of left forehead lesion including the orbital ridge and total decompression of the optic canal and superior orbital fissure improved these visual symptoms dramatically. Reconstructive cranioplasty using artificial bone made by hydroxyapatite (Apaceram) was very satisfactory in cosmetic appearance. Surgical indication and neuroradiological findings of cranial FD are discussed as well as a review of the literatures.

Adult↗
PubMed1979

A case of Albright's syndrome treated with calcitonin.

A 23-year-old woman with Albright's syndrome (polyostotic fibrous dysplasia of bone, precocious puberty and irregular cutaneous pigmentations) had sustained multiple fractures and was grossly disabled. Evaluation disclosed markedly raised serum alkaline phosphatases and a high urinary excretion of hydroxyproline, suggesting an accelerated bone turnover, while calcium metabolism was virtually undisturbed. During 12 months therapy with calcitonin, however, no apparent benefit was recorded and there was no evidence of any significant metabolic effects of the treatment. Initial discomfort with nausea and vomiting disappeared after dose reduction whereas diffuse bone and muscle pain, which gradually increased after a few months treatment, did not subside until after cessation of the therapy.

Adult↗
PubMed2005

Fibrous dysplasia of the cranial bones: a case report and review of the literature.

Fibrous dysplasia (FD) is a relatively uncommon disorder that affects primarily the cranial region; its occurrence in the cranial base in combination with hindbrain herniation and aneurysmal bone cyst (ABC) constitutes an extremely rare condition. We report a case of polyostotic fibrous dysplasia with progressive occipital, temporal, and clival involvement. Clinical findings and differential diagnosis with special emphasis on the imaging features were discussed. A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foramen magnum has been implicated in the development and subsequent progression of syringobulbia.

Adult↗
PubMed1986

The McCune-Albright syndrome: a lethal gene surviving by mosaicism.

In the McCune-Albright syndrome, fibrous dysplasia of bones and various forms of endocrine dysfunction are associated with multiple pigmented skin lesions. Examination of a 4-year-old female patient and comparison with photographs published in the literature revealed that the cutaneous pigmentation is arranged in a systematized pattern following the lines of Blaschko. Apparently, this pattern visualizes the dorso-ventral outgrowth of two different populations of cells during early embryogenesis. As all cases of the syndrome are sporadic, it is postulated that the disease is caused by an autosomal "dominant" lethal gene, leading to loss of the zygote in utero. Cells bearing the mutation can only survive when they are intermingled with normal cells. The mosaic may arise either from a gametic half chromatid mutation, or from an early somatic mutation. This concept offers an explanation for the scattered asymmetric distribution of bone lesions, and for the observation that the endocrinopathy may be either of central or peripheral origin, according to the random distribution of the mutant population of cells.

Child, Preschool↗
PubMed1978

[Fibrous dysplasia of the facial bones (author's transl)].

On the occasion of three cases of fibrous dysplasia (two of frontal sinus and the ethmoids and one of the upper jaw) the problem of fibrous dysplasia of the jaws is analysed. The clinical picture is characterized by a slow and gradual emergence of facial assymetric or exophathalmos. Histologically there is a variety of form. The treatment is largely a remodeling osteotomy. The writers effectively applied on all three cases a radial removal of the lesion.

Adolescent↗
PubMed1995

Quantitative analysis of the plain radiographic appearance of central chondrosarcoma of bone.

RATIONALE AND OBJECTIVE: To quantitate radiographic features that distinguish the plain radiographic appearance of central chondrosarcoma from other solitary bone lesions. MATERIALS AND METHODS: Seven hundred nine cases of focal bone lesions, including 37 central chondrosarcomas, were analyzed according to demographic, anatomic, and plain radiographic features. Vector analysis of groups of features was performed to determine those that are most sensitive and specific for the appearance of central chondrosarcoma in contrast with other lesions in the data base. RESULTS: The most specific appearance of long bone central chondrosarcomas was a lesion within the medullary cavity with geographic destruction, containing calcified matrix and a diameter greater than 5.9 cm, or arising within the proximal metaphysis or diaphysis. These features all are present in 9 (39%) of the 23 long bone central chondrosarcomas and in less than 1% of other lesions. In flat bone lesions, calcified matrix and either geographic bone destruction, medullary location, or enlargement of the host bone were 100% specific but of low sensitivity, identifying 7 (50%), 7 (50%), and 6 (41%) of the 14 flat bone chondrosarcomas, respectively. Inclusion of calcified matrix in the description is essential to make the criteria specific but excludes several of the most aggressive long bone chondrosarcomas. The vector analysis-generated differential diagnosis include osteosarcoma, fibrous dysplasia, unicameral bone cysts, aneurysmal bone cysts, malignant fibrous histiocytoma, and Ewing sarcoma. CONCLUSIONS: A relatively specific set of radiographic features can be defined to assist in plain film diagnosis of central chondrosarcoma, but there are many chondrosarcomas that fall outside these parameters. Thus, while not sufficiently sensitive to allow accurate plain film diagnosis in all cases, these criteria serve as an improved foundation for differential diagnosis.

Adolescent↗
PubMed2002

Aneurysmal bone cyst associated with fibrous dysplasia.

We report the clinicopathologic features of a 22-year-old patient with aneurysmal bone cyst and fibrous dysplasia of the orbit. The patient was evaluated clinically with computed tomography of the orbit before surgery. An orbital biopsy specimen was examined histologically with conventional light microscopy. The lesion was treated with combined neurosurgical and orbital intervention. Clinical evaluation revealed axial and inferior displacement of the globe. Computed tomography revealed a cystic mass in the superotemporal left orbit with adjacent bone erosion. "Ground-glass" thickening of the adjacent frontal bone and sphenoid bone was observed. Microscopic examination showed fibrous stroma with giant cells and hemosiderin-laden macrophages with adjacent trabeculae of woven bone and osteoblast cells. A fronto-orbital craniotomy was performed, the cystic cavity was excised, and the hyperostotic bone was debulked. After treatment, the globe position and patient appearance have improved. There has been no sign of recurrence of the aneurysmal bone cyst. Aneurysmal bone cyst should be considered in patients with fibrous dysplasia that has a cystic component, or in patients with fibrous dysplasia who present with sudden expansion of their lesion.

Adult↗